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Sweet syndrome in the setting of precedent bacteremia and inflammatory diseases (sicca syndrome and PBC), yet she presented with unusual giant cellulitis-like features. It is likely that giant cellulitis-like Sweet syndrome may be an uncommon clinical presentation for the different variants of Sweet syndrome and may not be restricted to the malignancy type. Edidiong Celestine Ntuen Kaminska, MD, MBS,a Adaobi I. Nwaneshiudu, MD, PhD,b Arlene Ruiz de Luzuriaga, MD, MPH,b Maria Tsoukas, MD, PhD,b and Diana Bolotin, MD, PhDb

Fig 1. Giant cellulitis-like Sweet syndrome. Initial presentation with erythematous, edematous, indurated papules and confluent plaques on the right back (superior) and buttock (inferior; intergluteal cleft marked by arrow).

Division of Dermatology, Department of Medicine, NorthShore University HealthSystem,a Skokie, Illinois, and Section of Dermatology, Department of Medicine, University of Chicago Medical Center,b Chicago, Illinois Funding sources: None. Conflicts of interest: None declared. Correspondence to: Edidiong Celestine Ntuen Kaminska, MD, MBS, Physician, Division of Dermatology, Department of Medicine, NorthShore University HealthSystem, 9933 Woods Drive, Skokie, IL 60077 E-mail: [email protected]

Fig 2. Giant cellulitis-like Sweet syndrome pathology. Prominent papillary dermal edema (top arrow) with abundant dermal neutrophils (bottom arrow).

plaques with bullous characteristics. Histopathology revealed classic findings of Sweet syndrome. Two patients were found to have an underlying malignancy (multiple myeloma or breast cancer); the other patient had no identified malignancy or autoimmune disease. To our knowledge, an extensive cellulitic presentation of Sweet syndrome has only been described in the 3 patients mentioned here. Like in these cases, our patient had severe widespread eruption of giant cellulitis-like plaques and was morbidly obese. However, to date, no malignancies have been identified in our patient. Because Sweet syndrome may herald onset or recurrence of malignancy,1,3 routine screenings and close follow-up are recommended. Our patient had classic

REFERENCES 1. Surovy AM, Pelivani N, Hegyi I, Buettiker U, Beltraminelli H, Borradori L. Giant cellulitis-like Sweet syndrome, a new variant of neutrophilic dermatosis. JAMA Dermatol 2013;149: 79-83. 2. Kroshinsky D, Alloo A, Rothschild B, Cummins J, Tan J, Montecino R, et al. Necrotizing Sweet syndrome: a new variant of neutrophilic dermatosis mimicking necrotizing fasciitis. J Am Acad Dermatol 2012;67:945-54. 3. Withrow RA, Shenenberger D, Kalish VB, Krivda S. Cellulitis unresponsive to antibiotics. Sweet’s syndrome. J Fam Pract 2011;60:149-51. 4. Dinh H, Murugasu A, Gin D. Sweet’s syndrome associated with cellulitis. Australas J Dermatol 2007;48:105-9. 5. Crum NF, Higginbottom PA, Fehl FC, Graham BS. Sweet’s syndrome masquerading as facial cellulitis. Cutis 2003;71:469-72. http://dx.doi.org/10.1016/j.jaad.2014.03.025

Cutaneous polyarteritis nodosa presenting as a solitary blue toe To the Editor: A woman in her 30s with a history of Crohn disease, quiescent on mesalamine, presented with a 1-year history of mild purple discoloration of the left great toe, which progressed to painful, burning nodules limiting movement of the digit. Her symptoms did not fluctuate with changing

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Fig 2. Cutaneous polyarteritis nodosa presenting as a solitary blue toe. Histopathologic specimen reveals a neutrophilic infiltrate obliterating a medium-sized blood vessel wall in the deep dermis consistent with a medium vessel vasculitis. (Hematoxylin-eosin stain; original magnification: 320.)

Fig 1. Cutaneous polyarteritis nodosa presenting as a solitary blue toe. Plantar side view of the left great toe reveals edema and violaceous nodularity with collarettes of thick scale and changes from the previous biopsy.

temperatures. She smoked less than 1 pack of cigarettes daily, but denied alcohol or drug use. Her only other medication was paroxetine. Numerous oral antibiotics and antifungals were previously ineffective. Prior arterial Doppler ultrasound was normal; magnetic resonance imaging demonstrated chronic degenerative arthritic changes. Physical examination demonstrated multiple tender subcutaneous nodules of the left hallux with background violaceous discoloration and edema limited to the toe (Fig 1). She was afebrile. Feet were warm with intact sensation and pulses. Punch biopsy showed medium vessel vasculitis (Fig 2) consistent with polyarteritis nodosa (PAN). Tissue cultures were negative. Complete blood count, complete metabolic panel, urinalysis, antinuclear antibody, rheumatoid factor, complements III/IV, human immunodeficiency virus, and hepatitis panels were normal. Prednisone 40 mg daily was initiated with disease resolution, but pain and edema returned at the end of the 6-week taper. Ibuprofen 600 mg every 6 hours was initiated with improvement. We present a case of cutaneous polyarteritis nodosa (cPAN) presenting as a solitary, painful, violaceous toe. Blue toe syndrome (BTS), named for the physical finding of a violaceous toe in the absence of trauma, cold-induced injury, or

generalized cyanosis, has numerous etiologies including emboli, thromboses, vasoconstriction, infections, and inflammatory disorders.1 Importantly, vasculitis can lead to BTS, including a case of granulomatosis with polyangiitis presenting as a single, tender, violaceous toe 5 months before the onset of any systemic symptoms.2 cPAN, considered a benign variant of polyarteritis nodosa (PAN), is frequently seen in association with inflammatory disorders, infections, and medications.3 It often presents as tender subcutaneous nodules on the lower extremities, with associated ulceration, livedo reticularis, petechiae, purpura, digital necrosis, and autoamputation. Women are commonly affected with a peak incidence in middle age.3 cPAN typically remains localized to the skin, follows a chronic, relapsing and remitting course, and, conventionally, does not progress to systemic PAN. If present, extracutaneous manifestations (arthralgias, myalgias, neuropathy) should remain localized to the region of affected skin.3 Inflammatory bowel disease has been reported in approximately 6% of cPAN cases,3 generally precedes the cPAN diagnosis, and is more likely associated with Crohn disease (CD) than ulcerative colitis.3,4 Flare in one disease does not predict poor control in the other.4 Since the initial report in 1970, fewer than 25 cases of cPAN associated with CD have been reported.3-5 Most recently, a report of 2 women with CD presented with subcutaneous nodules, edema, livedo reticularis, and Raynaud type symptoms. One woman had longstanding, quiescent CD before onset of cPAN, which required cyclophosphamide and ultimately led to amputation of a toe; the second had a banal cPAN


course controlled by a slow taper of prednisone with onset of symptoms around the time of her CD diagnosis. As in our patient, cPAN is generally responsive to corticosteroids.3,4 Nonsteroidal anti-inflammatory medications may be useful for prevention and treatment of mild flares and were successfully employed in our patient. We present an instructive case of cPAN presenting as a solitary blue toe to make clinicians aware of this unusual presentation in order to facilitate initiation of appropriate work-up and therapy. Amanda Joy Tschetter, MD,a Vincent Liu, MD,a,b and Karolyn A. Wanat, MDa,b Department of Dermatologya and Department of Pathologyb at the University of Iowa Hospitals and Clinics, Iowa City Funding sources: None. Conflicts of interest: None declared. Correspondence to: Amanda Joy Tschetter, MD, 200 Hawkins Drive, Iowa City, Iowa 52242 E-mail: [email protected] REFERENCES 1. Hirschmann JV, Raugi GJ. Blue (or purple) toe syndrome. J Am Acad Dermatol 2009;60:1-22. 2. Begon E, Bouilly P, Cheysson E, Cohen P, Bachmeyer C. Isolated blue toe syndrome as the initial sign of Wegener granulomatosis. Am J Med 2010;123:e7-8. 3. Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol 1997;136:706-13. 4. Komatsuda A, Kinoshita K, Togashi M, Maki N, Masai R, Niitsu H, et al. Cutaneous polyarteritis nodosa in a patient with Crohn’s disease. Mod Rheumatol 2008;18:639-42. 5. Magnant J, Lhommet C, Machet L, Machet MC, Guilmot JL, Diot E. [Cutaneous polyarteritis nodosa and Crohn’s disease: an association not to be ignored]. Rev Med Interne 2009;30: 345-8. http://dx.doi.org/10.1016/j.jaad.2014.03.037

Major response to vemurafenib in patient with severe cutaneous Langerhans cell histiocytosis harboring BRAF V600E mutation To the Editor: We report the case of a female patient in her 90s with a history of age-related macular degeneration and pulmonary embolism. In 2011, she presented with pruritic, erythematous, confluent, maculopapular skin lesions located on the trunk, inframammary fold, groin, and genital and perianal areas. Histology of a skin biopsy revealed a mononuclear cell infiltrate that was morphologically and phenotypically consistent with Langerhans cell

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histiocytosis (LCH) expressing CD1a and S100protein by immunohistochemistry. A body computed tomography scan showed no visceral involvement, the patient had no exophthalmos or diabetes insipidus, and laboratory tests related to LCH secondary localizations were normal (eg, inflammatory and hepatic markers, hemogram). Subsequently, a diagnosis of single system cutaneous LCH was established. Topical and systemic corticosteroid treatments failed to elicit any clinical improvement as first-line treatment, and the patient was suffering from chronic and severe pruritus and pain, affecting her quality of life that declined significantly. A second line of systemic treatment with thalidomide was initiated but was quickly discontinued because the patient experienced minor clinical response with major side effects such as dizziness, asthenia, and impaired coordination. Recently, she experienced a severe new flare of her disease with multiple papulonodular lesions in the same skin areas (Fig 1, before). The patient complained of major pain and had suicidal ideation related to her intolerable skin discomfort. No extracutaneous involvement was found, and a new biopsy confirmed the diagnosis of widespread single system cutaneous LCH (Fig 2, A-C). In conjunction with this finding, the DNA from the biopsy was extracted and a pyrosequencing analysis revealed a BRAF V600E mutation, as expected for up to 57% of LCH cases.1 After a multidisciplinary consultation, she was started on a third-line vemurafenib treatment, (960 mg orally twice a day) immediately after obtaining written informed consent. Vemurafenib induced a major clinical improvement in the first days of treatment. The patient was seen for follow-up 3 weeks after vemurafenib introduction and showed indisputable regression of her cutaneous lesions. Moreover, histopathologic and immunohistochemical studies confirmed the clinical response, in that a new biopsy revealed the absence of remaining cells expressing LCH markers (Fig 2, D and E). After the third month of treatment, the clinical response remained stable and after 6 months of vemurafenib, only scar tissue was noticeable (Fig 1, after). Vemurafenib is an anti-BRAF targeted therapy approved for the treatment of metastatic unresectable melanoma with V600 mutations; sensitivity to this molecule was recently demonstrated for BRAF mutated non-LCH (ie, CD1- negative ErdheimChester disease).2 Here we report a major clinical response to vemurafenib for refractory, widespread, skin-limited, pure LCH carrying the BRAF V600E

Cutaneous polyarteritis nodosa presenting as a solitary blue toe.

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